Autoimmune Hemolytic Anemia

Michel, Marc; Jäger, Ulrich

doi:10.1016/b978-0-323-35762-3.00046-9

Cited by 5 publications

(9 citation statements)

References 45 publications

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“…To avoid transfusion reactions and induction of alloantibodies following transfusions with ‘incompatible blood’, extended phenotyping of the donor RBCs is advisable in the majority of cases and an ‘ in vivo biological compatibility test’ is recommended in many countries. 23,33 In this test, approximately 20 ml of blood is given as a rapid infusion, the infusion is stopped for 20–30 min and, if no reaction occurs, the remaining infusion is administered at a normal rate. 23…”

Section: Waihamentioning

confidence: 99%

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Novel insights into the treatment of complement-mediated hemolytic anemias

Berentsen

Hill

et al. 2019

Therapeutic Advances in Hematology

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Complement-mediated hemolytic anemias can either be caused by deficiencies in regulatory complement components or by autoimmune pathogenesis that triggers inappropriate complement activation. In paroxysmal nocturnal hemoglobinuria (PNH) hemolysis is entirely complement-driven. Hemolysis is also thought to be complement-dependent in cold agglutinin disease (CAD) and in paroxysmal cold hemoglobinuria (PCH), whereas warm antibody autoimmune hemolytic anemia (wAIHA) is a partially complement-mediated disorder, depending on the subtype of wAIHA and the extent of complement activation. The pathophysiology, clinical presentation, and current therapies for these diseases are reviewed in this article. Novel, complement-directed therapies are being rapidly developed. Therapeutic terminal complement inhibition using eculizumab has revolutionized the therapy and prognosis in PNH but has proved less efficacious in CAD. Upstream complement modulation is currently being investigated and appears to be a highly promising therapy, and two such agents have entered phase II and III trials. Of these, the anti-C1s monoclonal antibody sutimlimab has shown favorable activity in CAD, while the anti-C3 cyclic peptide pegcetacoplan appears to be promising in PNH as well as CAD, and may also have a therapeutic potential in wAIHA.

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Section: Waihamentioning

confidence: 99%

“…23,33 In this test, approximately 20 ml of blood is given as a rapid infusion, the infusion is stopped for 20–30 min and, if no reaction occurs, the remaining infusion is administered at a normal rate. 23…”

Section: Waihamentioning

confidence: 99%

Novel insights into the treatment of complement-mediated hemolytic anemias

Berentsen

Hill

et al. 2019

Therapeutic Advances in Hematology

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“…3,4 Increasingly, pharmacological treatment is considered in patients with symptomatic anemia, severe cold-induced circulatory symptoms, and/or thrombosis. 20,30,32,56,57 Unspecific and supportive therapies At least in some countries, therapy with corticosteroids remains frequently used in clinical practice. 51,57 No trials have been published, but retrospective series have described low response rates and heterogeneous and weak response criteria.…”

Section: Watchful Waiting and Non-pharmacological Managementmentioning

confidence: 99%

“…3,55 It is, therefore, widely agreed that corticosteroids should not be used to treat CAD. 30,32,55,56,58,59 Other unspecific immune suppression, for example, with azathioprine, has not proved beneficial. 3,60 Therapy with interferon-α was ineffective in a cohort of four patients although some conflicting evidence does exist.…”

Section: Watchful Waiting and Non-pharmacological Managementmentioning

confidence: 99%

“…30,32,34 The terms CAD (or cold hemagglutinin disease, CHAD) and CAS (or cold hemagglutinin syndrome, CHAS) have previously been used interchangeably, but these entities should be distinguished and defined as explained in the "Introduction" section. 9,20,56 The criteria for CR and PR used in this review are identical to those applied in most prospective trials of B-cell-directed therapies for CAD, 22,66,68,71 but these response definitions are not applicable to treatment with complement inhibitors. 16,20,80 Literature-or consensus-based definitions would be appreciated.…”

Section: Challenges and Future Perspectives Concepts And Definitionsmentioning

confidence: 99%

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<p>Cold agglutinin disease: current challenges and future prospects</p>

Berentsen¹,

Roth²,

Randen³

et al. 2019

JBM

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Cold agglutinin disease (CAD) is a complement-dependent, classical pathway-mediated immune hemolytic disease, accounting for 15–25% of autoimmune hemolytic anemia, and at the same time, a distinct clonal B-cell lymphoproliferative disorder of the bone marrow. The disease burden is often high, but not all patients require pharmacological treatment. Several therapies directed at the pathogenic B-cells are now available. Rituximab plus bendamustine or rituximab monotherapy should be considered first-line treatment, depending on individual patient characteristics. Novel treatment options that target the classical complement pathway are under development and appear very promising, and the C1s inhibitor sutimlimab is currently being investigated in two clinical Phase II and III trials. These achievements have raised new challenges and further prospects, which are discussed. Patients with CAD requiring therapy should be considered for clinical trials.

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